Contents
Introduction
↑ Pressure in portal venous system. (> 12 mmHg)
- Normal portal pressure: 5-10 mmHg
- Liver receives 25% cardiac output through dual vascular supply:
- Portal venous circulation: 75–80% blood supply (low-pressure system)
- Hepatic artery: 20-25% (high-pressure system)
Aetiology
Prehepatic (portal vein):
- Portal venous thrombosis
- Extrahepatic portal venous obstruction (EHPVO) (M/C cause, 50-75%)
- Cavernous transformation of portal vein
- Isolated splenic vein thrombosis
Intrahepatic (liver):
- Liver cirrhosis (2nd M/C, 25-35%)
- Non-cirrhotic portal fibrosis (NCPF) (10-15%)
- Congenital hepatic fibrosis
- Veno-occlusive disease
- Noncirrhotic portal fibrosis
- Schistosomiasis
- Sarcoidosis
- Nodular regenerative hyperplasia
Posthepatic (heart, CVC, hepatic Veins):
- Budd-Chiari syndrome (hepatic vein or inferior vena cava obstruction) (8–26%)
- Constrictive pericarditis
- Right-sided heart failure
Pathophysiology

Clinical features
- GI bleed (M/C presentation):
- M/C d/t oesophagal varices
- Ascites (hepatic decompensation)
- Caput medusae
- Diminished liver function
- Splenomegaly
Complications
Ascites (free fluid in the peritoneal cavity):
PH → Endothelial NO release in blood vessels → Hypotension → Compensatory aldosterone release (kidneys) → Increased water retention → Pooling of blood
Dilutional (hypervolemic) hyponatremia:
PH → Endothelial NO release in blood vessels → Hypotension → Compensatory aldosterone release (kidneys) → Increased water retention → Hyponatremia
- Marker of late-stage disease and a negative prognostic indicator
Hepatorenal syndrome:
Reversible renal failure as a consequence of profound renal vasoconstriction secondary to the release of angiotensin, norepinephrine, and ADH in response to splanchnic vasodilatation
Pulmonary complications:
Microvascular pulmonary arterial dilatation (most likely because of NO overproduction in the lung) leading to ventilation‐perfusion mismatch.
- Hepatopulmonary syndrome:
- Triad:
- Chronic liver disease/portal hypertension
- Alteration of arterial oxygenation
- Intrapulmonary vascular dilatations
- Triad:
- Portopulmonary syndrome
- Pulmonary arterial hypertension (>25 mmHg)
- Hepatic hydrothorax: Pleural effusion with hepatobiliary disease
- Because of direct passage of ascites from abdomen to thorax through undetectable diaphragmatic rent
Spontaneous bacterial peritonitis (8–30% in cases with ascites):
- Mortality: 20–40% if untreated
- Asymptomatic or abdominal pain, fever, and diarrhea
- Neutrophil >250 cells/mm3 in ascitic fluid (DIAGNOSTIC, regardless of pathogen detection)
Hypersplenism:
Congestive splenomegaly and ↑ hemolysis by the enlarged spleen.
- Pancytopenia
Portal hypertensive gastropathy:
Mucosal and submucosal vascular ectasia in the absence of inflammation.
- Impairment of gastric mucosal defence
- Thrombosis of mesenteric vessels (d/t circulatory stasis)
- Alterations in gastrointestinal motility
- Bacterial overgrowth (lack of intestinal bile acids)
- Mucosal oedema (↑ gastrointestinal mucosal permeability)
Acquired portosystemic shunts:
- Anorectal varices: May bleed and cause blood in stool: Hematochezia
- Oesophageal varices: May bleed and cause vomiting of blood: Haematemesis
- Caput medusae: Swollen veins on the anterior abdominal wall
- Haemorrhoids: Swollen or dilated hemorrhoidal veins
Hepatic encephalopathy (neurocognitive impairment):
↓ Liver function & ↓ blood detoxification → Toxic metabolites cross BBB
- Clinically manifested as a range of signs from subtle behavioural deficits to stupor and coma.
Liver failure:
- Palmar erythema
- Gynecomastia
- Spider naevi
- Loss of axillary and pubic hair
Diagnosis
Hepatic venous pressure gradient (HVPG) (GOLD STANDARD):
Assess severity of portal hypertension. HVPG is calculated as the difference between the wedged hepatic vein pressure (WHVP) and free hepatic vein pressure (FHVP).
- Portal hypertension is defined as HVPG ≥ 5 mmHg
- Clinically significant when > 10-12 mmHg

Imaging:
- Ultrasonography (USG)
- First-line imaging technique for the diagnosis and follow-up
- Findings:
- Dilated portal vein (diameter > 13 or 15 mm)
- Doppler ultrasonography
- Slow velocity of <16 cm/s + to dilatation in the MPV (DIAGNOSTIC)

Management
- Beta-blockers (propranolol):
- ↓ Portal venous pressure
- Prevent complications
Ascites:
- Sodium restriction
- Diuretics (↓ fluid overload)
Bleeding oesophageal varices (MEDICAL EMERGENCY):
- Octreotide
- Balloon tamponade
- Sclerotherapy
- Variceal ligation
Transjugular intrahepatic portal systemic shunt (TIPS):
Introduced through the right internal jugular vein, catheterization of a main hepatic vein, transparenchymal catheterization of the portal vein, and serial dilation of the track until large enough to insert a stent. Once placed the stent is dilated sufficiently to reduce the portal-systemic gradient below 12 mmHg.
- Success rate: 92–99%
- Absolute contraindications: Right-sided heart failure, severe liver failure and polycystic liver disease
- Relative contraindications: Hepatic tumours, encephalopathy and hepatic/portal vein thrombosis
- Complications: Acute stent thrombosis, hemobilia, stent migration and others related to angiography in general
